Health-related Quality of Life of Children Treated for Non-syndromic Craniosynostosis

Abstract

Background: Assessing health-related quality of life (HRQoL) allows acquisition of the subjective perspective of patients regarding their health and functioning; however, little is known about the experiences of patients living with treated craniosynostosis (CS).

Methods: School-aged children (7–16 years) treated for non-syndromic CS were assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales via both self- and proxy reports.

Results: Seventy-three patients and their parents responded to the PedsQL (response rate: 80.2%). Patients generally estimated HRQoL as high, with no difference in HRQoL found between treated sagittal (SS) or metopic (MS) synostosis. In the SS group, surgical methods involving spring-assisted surgery and pi-plasty were unrelated to HRQoL outcomes. Additionally, HRQoL was highly correlated with intelligence quotient (IQ) and adaptive behavior skills (ABAS). Furthermore, we observed differences in estimated HRQoL between self- and proxy reports (i.e., parents estimated child HRQoL as higher than did the children).

Conclusions: Children treated for CS have a generally high HRQoL, and neither CS type nor surgical method influenced HRQoL outcomes. Moreover, children and parents estimated HRQoL differently, suggesting the importance of using both self- and proxy reporting in patient-reported measures. We found that HRQoL was strongly related to IQ and ABAS, indicating that the PedsQL can be used as a screening instrument to identify craniofacial patients in need of further psychological assessment.

Introduction

In recent years, health-related quality of life (HRQoL) has become increasingly important as a tool for providing patient perspectives regarding their health; however, the relationship between health and HRQoL is not always consistent, as having a disease does not always negatively impact perceived QoL, and health does not always guarantee a better QoL.¹

The definition of HRQoL is multifaceted, with several definitions existing in the literature. The World Health Organization definition of QoL is described as the perception by an individual of their position in life regarding their physical health, psychological state, level of independence, and social relationships.² HRQoL represents a multidimensional concept that also includes health status and its impact on well-being or QoL. The Pediatric Quality of Life Inventory (PedsQL) was developed to measure HRQoL associated with pediatric health care by James Varni in the late 1990s and covers important domains, including physical, psychological, and social functioning, with the recent addition of school functioning.^3–5

Treatment outcomes for non-syndromic craniosynostosis (CS) have been studied from multiple perspectives, with numerous previous studies assessing cognitive, behavioral, and emotional functioning and the impact of surgical methods or timing on development.^6–22 However, little is known about the relationship between CS and HRQoL, despite previous claims that QoL has been included as part of psychosocial assessment. In fact, few previous studies have used validated instruments for the purpose of measuring the HRQoL of treated CS patients.^23–25 Previous studies report a high QoL in adult patients,²³ whereas another study of children treated for non-syndromic CS revealed an elevated risk of lower HRQoL.²⁴ Additionally, children with untreated sagittal synostosis (SS) score low in terms of positive emotions.²⁵

The aim of this study was to evaluate the HRQoL of children treated for non-syndromic CS and using the Swedish version of the validated PedsQL 4.0 Generic Core Scales.

Patients And Methods

Patients and Procedures

Children (aged 7–16 years) treated for non-syndromic CS and living in the regions of Västra Götaland and Halland in Sweden were included in this study. The patients were initially informed of the study by letter, and after 1 week, received a telephone call with additional information about the study and invited to participate by scheduling a visit to the clinic. Background data were extracted from the Gothenburg Craniofacial Registry and previous studies of the same patient cohort.^13,26

Measurements Using PedsQL 4.0 Generic Score Scales (Swedish version)

PedsQL is a modular questionnaire that measures the HRQoL of children ages 2 to 18 years. The questionnaire comprises 23 items measuring four dimensions of HRQoL: physical (8 items), emotional (5 items), social (5 items), and school functioning (5 items). PedsQL comprises both self- and proxy reports and has four age- and language-appropriate versions (2–4 years, 5–7 years, 8–12 years, and 13–18 years).

The questionnaire asks about difficulties with functions during the previous month. The response alternatives are presented on a 5-point scale: 0, never a problem; 1, almost never a problem; 2, sometimes a problem; 3, often a problem; and 4, almost always a problem. The response alternatives are converted to an overall scale (0–100 points) and reversed, so that higher points indicate better HRQoL.

The Swedish version of PedsQL has been translated and validated as demonstrating good psychometric properties.²⁷ Additionally, the instrument has been evaluated in large groups of Swedish school children (n = 1455).²⁸ In the present study, we used both self- and proxy reports, and children and parents responded separately.

Statistical Analysis

Comparison of background variables between two groups was conducted using Fisher’s exact test for dichotomous variables, a Mann–Whitney U test for continuous variables, and a chi-squared test for unordered categorial variables. Comparisons within groups (the study groups vs. norms and self- vs. proxy reports) were analyzed using Fisher’s non-parametric permutation test for matched pairs. Between-group comparisons were performed using Fisher’s non-parametric permutation test for continuous variables. Adjustment for confounders was analyzed by logistic regression. Calculation of 95% confidence intervals for mean differences was performed using Fisher’s non-parametric permutation test between groups. The relationship between HRQoL (PedsQL), adaptive behavior skills (ABAS), and intelligence quotient (IQ) was analyzed by Spearman’s correlation coefficient (r_s). All significance tests were two-sided, with a p < 0.05 considered significant. All statistical calculations were performed using SAS (v.9.4; SAS Institute, Cary, NC, USA).

Ethics

The study was approved by the Gothenburg Ethics Committee (no. 856 − 13) and conducted according to principles in the Declaration of Helsinki.

Results

Attrition Analysis

Attrition analysis revealed no significant differences between the responding (n = 73) and non-responding (n = 18) groups regarding background variables, including gender, age at study, CS type, surgical method, age at surgery, and premature birth (Table 1).

Table 1

Attrition analysis.
Variables	Non-participating (n = 18)	Participating (n = 73)	p
Sex
Female	6 (33.3%)	24 (32.9%)
Male	12 (66.7%)	49 (67.1%)	1.00
Age at study	11.7 (2.2) 12 (8; 15) n = 18	11.0 (2.4) 11 (7; 15) n = 73	0.23
Age at surgery (days)	277.1 (338.6) 159.5 (94; 1523) n = 18	237.0 (224.7) 165 (84; 1484) n = 73	0.74
Craniosynostosis
Sagittal	9 (50.0%)	41 (56.2%)
Metopic	6 (33.3%)	24 (32.9%)
Unicoronal	1 (5.6%)	6 (8.2%)
Bicoronal	1 (5.6%)	1 (1.4%)
Lambdoid	1 (5.6%)	1 (1.4%)	0.64
Surgery method
Pi-plasty	1 (5.6%)	17 (23.3%)
Spring-assisted surgery	8 (44.4%)	23 (31.5%)
Fronto-orbital reshaping with bone graft	6 (33.3%)	14 (19.2%)
Fronto-orbital reshaping with spring	3 (16.7%)	18 (24.7%)
Barrel-stave osteotomy	0 (0.0%)	1 (1.4%)	0.29
Born premature
Yes	3 (17.6%)	5 (6.8%)
No	14 (82.4%)	68 (93.2%)	0.34
For categorical variables, n (%) is presented. For continuous variables, mean (SD) / median (min; max) / n is presented. For comparison between groups, Fisher´s Exact test (lowest 1-sided p-value multiplied by 2) was used for dichotomous variables, a chi-squared test was used for non-ordered categorical variables, and the Mann–Whitney U test was used for continuous variables.

Participants

A total of 73 patients (24 females and 49 males; response rate: 80.2%) and their parents participated in the study. The mean age at study was 11.0 ± 2.4 years (range: 7–15 years), and all patients were treated for non-syndromic CS [SS, n = 41; metopic (MS), n = 24; and other, n = 8]. Mean age at surgery was 237.0 days, and the mean IQ (98.1) and ABAS (94.4) were within average ranges (Table 2).

Table 2

Demographics and Baseline Characteristics.
Variables	Total (n = 73)
Sex
Female	24 (32.9%)
Male	49 (67.1%)
Age at study	11.0 (2.4) 11 (7; 15) n = 73
Craniosynostosis
Sagittal	41 (56.2%)
Metopic	24 (32.9%)
Unicoronal	6 (8.2%)
Bicoronal	1 (1.4%)
Lambdoid	1 (1.4%)
Surgery method
Pi-plasty	17 (23.3%)
Spring-assisted surgery	23 (31.5%)
Fronto-orbital reshaping with bone graft	14 (19.2%)
Fronto-orbital reshaping with spring	18 (24.7%)
Barrel-stave osteotomy	1 (1.4%)
Age at surgery (days)	237.0 (224.7) 165 (84; 1484) n = 73
Involved parent
Mother	36 (50.0%)
Father	12 (16.7%)
Both	24 (33.3%)
Maternal education level
Primary school	2 (3.4%)
High school	21 (35.6%)
College/University	36 (61.0%)
Paternal education level
Primary school	2 (5.7%)
High school	19 (54.3%)
College/University	14 (40.0%)
Born premature
Yes	5 (6.8%)
No	68 (93.2%)
Did the child have other diseases/diagnosis?
Yes	22 (30.6%)
No	50 (69.4%)
What kind of condition?
Heart disease	4 (23.5%)
Neuropsychiatric diagnosis	5 (29.4%)
Asthma/Allergy	4 (23.5%)
Migraine	3 (17.6%)
Metabolic disease	1 (5.9%)
Has your child been assessed by a psychologist?
Yes	14 (19.2%)
No	59 (80.8%)
What did the psychological assessment show?
Autism	4 (30.8%)
Problem with executive functions	1 (7.7%)
ADHD	2 (15.4%)
Problem with attention	2 (15.4%)
Both autism and ADHD	2 (15.4%)
Development delay	2 (15.4%)
Is your child under medical treatment?
Yes	8 (11.4%)
No	62 (88.6%)
Right- or left-handed?
Right	62 (87.3%)
Left	9 (12.7%)
Wechsler Full Scale Intelligence Quotient	98.1 (14.6) 100 (59; 135) n = 72
Wechsler Verbal Comprehension Intelligence Quotient	99.5 (14.6) 100 (57; 130) n = 72
Wechsler Perceptual Reasoning Intelligence Quotient	104.5 (13.7) 102 (69; 135) n = 72
Wechsler Working Memory Intelligence Quotient	94.9 (13.0) 97 (56; 120) n = 71
Wechsler Processing Speed Intelligence Quotient	92.1 (14.4) 91 (53; 126) n = 71
Adaptive Behaviour Skills Full Scale	94.4 (20.2) 97.5 (47; 120) n = 72
Conceptual Composite Scale	93.9 (20.1) 95.5 (40; 119) n = 72
Social Composite Scale	93.4 (19.6) 97 (42; 118) n = 72
Practical Composite Scale	94.6 (19.8) 97 (55; 120) n = 72
For categorical variables, n (%) is presented. For continuous variables, mean (SD) / median (min; max) / n is presented.

Health-related QoL of Patients Treated for CS Relative to Controls

The HRQoL of treated CS patients was estimated as generally high, with no differences found on a full-scale level relative to controls. At a detailed level, the SS group reported significantly lower school functioning (p = 0.002) and psychosocial functioning (p = 0.031) as compared with normal population. The MS group reported a high HRQoL, with no significant difference relative to controls (Table 3).

Table 3

Child-rated PedsQL Generic Core Scales Relative to Norms.
	Craniosynostosis (n = 73)		Sagittal (n = 41)		Metopic (n = 24)
Variables	Mean (SD) Median (Min; Max) n	p (within group)	Mean (SD) Median (Min; Max) n	P (within group)	Mean (SD) Median (Min; Max) n	p (within group)
HRQoL PedsQL Full Scale	−0.181 (1.137) − 0.04 (− 4.573; 1.241) n = 67	0.20	−0.262 (1.107) − 0.089 (− 3.489; 1.044) n = 40	0.14	−0.237 (1.259) − 0.04 (− 4.573; 1.241) n = 21	0.45
Physical Functioning Scale	0.101 (0.906) 0.141 (− 3.803; 1.069) n = 69	0.36	0.089 (0.872) 0.141 (− 1.947; 1.069) n = 40	0.54	−0.020 (1.015) 0.141 (− 3.803; 1.069) n = 23	0.94
Emotional Functioning Scale	−0.062 (0.996) 0.087 (− 3.001; 1.117) n = 69	0.61	−0.102 (0.970) 0.259 (− 3.001; 1.117) n = 40	0.51	−0.092 (1.044) 0.087 (− 3.001; 1.117) n = 23	0.69
Social Functioning Scale	−0.274 (1.312) 0.28 (− 5.115; 0.695) n = 69	0.082	−0.374 (1.348) 0.28 (− 5.115; 0.695) n = 40	0.085	−0.298 (1.375) 0.28 (− 4.285; 0.695) n = 23	0.34
School Functioning Scale	−0.514 (1.316) − 0.342 (− 4.046; 1.139) n = 67	0.0014	−0.676 (1.370) − 0.342 (− 4.046; 1.139) n = 40	0.0018	−0.430 (1.314) − 0.342 (− 4.046; 1.139) n = 23	0.14
Psychosocial Functioning Scale	−0.333 (1.281) 0.001 (− 4.504; 1.202) n = 67	0.035	−0.454 (1.284) − 0.225 (− 4.354; 1.202) n = 40	0.031	−0.321 (1.368) − 0.15 (− 4.504; 1.202) n = 23	0.32
For continuous variables, mean (SD) / median (min; max) / n is presented. For comparison within groups, Fisher´s non-parametric permutation test for matched pairs was used. For each variable, the z-score was calculated as (PedsQL − normal mean) / normal SD.

The Impact of CS Type

Background data variables “age at study” and “working memory IQ” differed between the SS and MS groups, with this adjusted for prior to the analysis. No differences were found in HRQoL outcomes between the SS and MS groups (Table 4).

Table 4

Difference in the mean of child- and parent-rated PedsQL Generic Core Scales between treated sagittal and metopic CS patients.
Variables	Sagittal (n = 41)	Metopic (n = 24)	p	Adjusted p*	Difference between groups^a	Effect size^b
HRQoL PedsQL Full Scale	85.4 (9.8) 86.4 (53.3; 98.4) n = 39	85.8 (11.2) 87.5 (52.2; 97.8) n = 21	0.90		−0.412 (− 6.095; 5.054)	−0.040
Physical Functioning Scale	89.5 (8.0) 90.6 (64.1; 100) n = 40	87.8 (11.7) 89.1 (39.1; 98.4) n = 23	0.53	0.82	1.72 (− 3.37; 6.39)	0.181
Emotional Functioning Scale	81.7 (11.8) 81.3 (55; 100) n = 40	83.4 (11.8) 85 (52.5; 97.5) n = 23	0.61		−1.68 (− 7.92; 4.42)	−0.142
Social Functioning Scale	89.7 (12.0) 92.5 (37.5; 100) n = 39	88.4 (14.1) 92.5 (42.5; 100) n = 23	0.70		1.31 (− 5.67; 7.69)	0.102
School Functioning Scale	77.6 (17.7) 81.3 (35; 100) n = 40	83.0 (14.2) 87.5 (47.5; 97.5) n = 21	0.24	0.76	−5.41 (− 14.55; 3.39)	−0.326
Psychosocial Functioning Scale	83.3 (11.9) 85.8 (47.5; 97.5) n = 39	84.8 (12.5) 86.7 (51.7; 98.3) n = 21	0.66		−1.53 (− 8.12; 4.88)	−0.127
For continuous variables, mean (SD) / median (min; max) / n is presented. For comparison between groups, Fisher´s non-parametric permutation test was used for continuous variables. ^a Data represent the mean (95% CI). The CI for the difference between groups is based on Fisher’s non-parametric permutation test. ^b Data represent the difference in the mean / pooled SD. * Adjustment of physical functioning for age at study and school functioning for WMIQ was performed using logistic regression. CI, confidence interval.

The Impact of Surgical Method Used to Treat SS

Comparisons of SS treated with spring-assisted surgery and pi-plasty revealed no significant differences, indicating that the effect of the surgical method used to treat SS was unrelated to HRQoL outcomes (Table 5).

Table 5

Difference in the mean of child- and parent-rated PedsQL Generic Core Scales between pi-plasty and spring-assisted surgery used to treat patients with sagittal CS.
Variables	Pi-plasty (n = 17)	Spring-assisted surgery (n = 23)	p	Difference between groups^a	Effect size^b
HRQoL PedsQL Full Scale	84.9 (11.7) 88.3 (53.3; 97.8) n = 16	85.6 (8.6) 86.4 (67.4; 98.4) n = 22	0.83	−0.738 (− 7.405; 5.978)	−0.074
Physical Functioning Scale	89.2 (10.0) 89.1 (64.1; 100) n = 17	90.1 (6.4) 91.4 (76.6; 100) n = 22	0.75	−0.902 (− 6.250; 4.492)	−0.111
Emotional Functioning Scale	82.1 (13.6) 82.5 (55; 100) n = 17	81.4 (10.8) 80 (57.5; 95) n = 22	0.89	0.695 (− 7.187; 8.750)	0.057
Social Functioning Scale	87.2 (15.0) 92.5 (37.5; 100) n = 16	91.1 (9.4) 92.5 (65; 100) n = 22	0.38	−3.95 (− 11.79; 4.06)	−0.328
School Functioning Scale	77.4 (18.0) 80 (42.5; 95) n = 17	77.3 (18.2) 81.3 (35; 100) n = 22	1.00	0.080 (− 11.667; 12.187)	0.004
Psychosocial Functioning Scale	82.9 (13.2) 86.7 (47.5; 96.7) n = 16	83.3 (11.4) 84.2 (54.2; 97.5) n = 22	0.94	−0.341 (− 8.417; 7.812)	−0.028
For continuous variables, mean (SD) / median (min; max) / n is presented. For comparison between groups, Fisher´s non-parametric permutation test was used for continuous variables. ^a Data represent the mean (95% CI). The CI for the difference between groups is based on Fisher’s non-parametric permutation test. ^b Data represent the difference in the mean / pooled SD. CI, confidence interval.

HRQoL Correlates with IQ and ABAS

HRQoL significantly correlated with IQ (r = 0.57; p = 0.0001) and ABAS (r = 0.42; p = 0.0004) (Table 6).

Table 6

Correlation analysis of the mean of child- and parent-rated PedsQL Generic Core Scales with ABAS and IQ.
Variables	HRQoL PedsQL Full scale	Physical Functioning Scale	Emotional Functioning Scale	Social Functioning Scale	School Functioning Scale	Psychosocial Functioning Scale
Adaptive Behaviour Skills Full Scale	0.57 < 0.0001 65	0.32 0.0084 68	0.50 < 0.0001 68	0.40 0.0008 67	0.55 < 0.0001 66	0.57 < 0.0001 65
Conceptual Composite Scale	0.57 < 0.0001 65	0.33 0.0053 68	0.46 < 0.0001 68	0.38 0.0013 67	0.57 < 0.0001 66	0.57 < 0.0001 65
Social Composite Scale	0.52 < 0.0001 65	0.38 0.0015 68	0.52 < 0.0001 68	0.35 0.0038 67	0.44 0.0002 66	0.52 < 0.0001 65
Practical Composite Scale	0.53 < 0.0001 65	0.28 0.022 68	0.46 < 0.0001 68	0.37 0.0020 67	0.52 < 0.0001 66	0.53 < 0.0001 65
Wechsler Full Scale Intelligence Quotient	0.42 0.0004 65	0.16 0.19 68	0.28 0.022 68	0.34 0.0046 67	0.47 < 0.0001 66	0.42 0.0004 65
Wechsler Verbal Comprehension Intelligence Quotient	0.39 0.0014 65	0.26 0.030 68	0.26 0.036 68	0.30 0.013 67	0.40 0.0009 66	0.39 0.0014 65
Wechsler Perceptual Reasoning Intelligence Quotient	0.31 0.013 65	0.05 0.66 68	0.18 0.13 68	0.22 0.069 67	0.34 0.0047 66	0.31 0.013 65
Wechsler Working Memory Intelligence Quotient	0.20 0.12 65	0.06 0.60 68	0.14 0.27 68	0.16 0.20 67	0.29 0.019 66	0.20 0.12 65
Wechsler Processing Speed Intelligence Quotient	0.36 0.0030 65	-0.01 0.93 68	0.19 0.12 68	0.33 0.0065 67	0.43 0.0003 66	0.36 0.0030 65
For each variable, Spearman’s correlation is presented with the corresponding p-value and the number of observations used.

Conformity of Self- and Proxy Reports

The conformity of self- and proxy reports was high in regard to emotional functioning; however, we observed a significant difference in estimated HRQoL (p = 0.031), physical functioning (p = 0.002) and school functioning (p = 0.012), for which the parents estimated better functioning than the children (Table 7).

Table 7

Change in PedsQL Generic Core Scales from child rater to parent rater.
	Child (n = 73)	Parent (n = 73)	Change from child to parent (n = 73)
Variable	Mean (SD) Median (Min; Max) (95% CI using the inversion of Fisher´s non-parametric permutation test), n	Mean (SD) Median (Min; Max) (95% CI using the inversion of Fisher´s non-parametric permutation test), n	Mean (SD) Median (Min; Max) (95% CI using the inversion of Fisher´s non-parametric permutation test), n SRM ES	p (within group)
HRQoL PedsQL Full Scale	84.3 (12.5) 85.9 (35.9; 100) (81.3; 87.3) n = 67	88.0 (11.4) 90.8 (58.7; 100) (85.2; 90.7) n = 68	3.61 (13.15) 3.8 (− 19.57; 33.7) (0.40; 6.84) n = 66 0.27 0.29	0.031
Physical Functioning Scale	87.0 (12.2) 87.5 (34.4; 100) (84.0; 89.8) n = 69	92.2 (10.9) 96.9 (43.8; 100) (89.6; 94.8) n = 69	5.25 (13.53) 3.13 (− 21.88; 37.5) (2.00; 8.52) n = 69 0.39 0.43	0.0022
Emotional Functioning Scale	82.8 (14.5) 85 (40; 100) (79.3; 86.2) n = 69	82.5 (15.4) 85 (40; 100) (78.9; 86.2) n = 69	−0.290 (18.921) 0 (− 40; 60) (− 4.833; 4.242) n = 69 − 0.02 − 0.02	0.94
Social Functioning Scale	88.3 (15.8) 95 (30; 100) (84.6; 92.1) n = 69	91.4 (13.2) 97.5 (35; 100) (88.2; 94.6) n = 68	2.87 (15.34) 0 (− 30; 45) (− 0.78; 6.56) n = 68 0.19 0.18	0.14
School Functioning Scale	77.7 (17.8) 80 (30; 100) (73.4; 82.0) n = 67	83.0 (19.6) 90 (30; 100) (78.4; 87.7) n = 69	5.82 (18.14) 10 (− 50; 60) (1.41; 10.30) n = 67 0.32 0.33	0.012
Psychosocial Functioning Scale	83.0 (14.2) 86.7 (36.7; 100) (79.5; 86.4) n = 67	85.8 (13.7) 90 (46.7; 100) (82.5; 89.1) n = 67	2.60 (14.98) 3.33 (− 33.33; 45) (− 1.03; 6.26) n = 66 0.17 0.18	0.17
For continuous variables, mean (SD) / median (min; max) / (95% CI using the inversion of Fisher´s non-parametric permutation test) / n is presented. For comparison within groups, Fisher´s non-parametric permutation test for matched pairs was used. Change was calculated, as follows: difference = parent rate − child rate. SRM, standardized response mean = mean difference / SD of the difference. ES, effect size = mean difference / SD for child. CI, confidence interval.

Discussion

In this study of children treated for non-syndromic CS, we asked patients and their parents to estimate HRQoL using the PedsQL 4.0 Generic Core Scales as a reliable and validated HRQoL-measurement tool specifically constructed for children in pediatric care. Measuring HRQoL with a patient-reported instrument allows access to information directly from the perspective of the patient, which remains a rare but coveted phenomenon in both research and clinical settings. There are few studies examining the subjective perception of the HRQoL of patients treated for non-syndromic CS, with only one study focusing on children treated for CS and reporting results indicating a risk of low HRQoL.²⁴ In another study of untreated SS patients, there was a tendency toward a low score in relation to positive emotions.²⁵

This study, which included a cohort of 73 patients treated for non-syndromic CS, revealed a generally high HRQoL, with no significant differences identified between those treated for CS and norms or between those treated for SS and MS. However, reports of psychosocial and school functioning were lower in the SS group, although no differences in functioning were found between the SS and MS groups according to the estimated HRQoL. Furthermore, the surgical method used for treatment (i.e., spring-assisted surgery and pi-plasty) in the SS group was unrelated to better or worse HRQoL outcomes.

Numerous studies have focused on assessing neuropsychological and cognitive functioning in order to reach a consensus regarding developmental impacts related to CS diagnosis and surgical treatment.^{6–9, 11-20,22} Previous studies indicate that non-syndromic CS patients are generally expected to exhibit average cognitive development.^{11,13,15,17,29} The cohort of patients in the present study showed average performance in terms of IQ and ABAS, which were extracted as background data from previous studies.^13,26 Notably, we found that associations between HRQoL, IQ, and ABAS were significant, with moderate correlations. This is an important aspect to consider when measuring HRQoL, given that cognitive and adaptive abilities can affect HRQoL outcomes. Therefore, it is crucial to control for these variables in order to measure the intended phenomenon. In a clinical setting, HRQoL could be used to screen patients in need of further psychological assessment.³⁰

Patient-reported outcomes can offer information regarding patient perspective through self- or proxy reports. In this study, we used both self- and proxy reports to assess HRQoL. Interestingly, there were differences in how children and parents estimated HRQoL, with parents inclined to report higher HRQoL than the children. Previous studies frequently used proxy reports, where parents were asked to assess different aspects of developmental questions regarding their child.^10,31−34 The results of the present study indicate that it is critical to also use self-reports before drawing conclusions about patient status.

The primary strength of this study is its methodological approach using a validated and reliable measurement, evaluated in a larger group of Swedish schoolchildren, to assess HRQoL. Another methodological strength is the advantages of controlling for confounders through the use of comprehensive amounts of background data. Additionally, the high response rate (80.2%) and the attrition analysis minimized the risk of selection bias. However, there are also limitations, as a larger study group would have increased the probability of the assumptions from the results.

Conclusions

In summary, these results concluded that children treated for non-syndromic CS have a generally high HRQoL, with neither CS type nor surgical method used in the SS group related to better or worse HRQoL outcomes. Moreover, we suggest that measuring HRQoL in a clinical setting can be used as a screening method to detect patients in need of profound psychological assessment. Furthermore, using both self- and proxy reports is crucial, given that parents tend to overestimate the HRQoL of their child.

List of abbreviations

Adaptive behavior skills (ABAS)

Craniosynostosis (CS)

Health-related quality of life (HRQoL)

Intelligence quotient (IQ)

Metopic synostosis (MS)

Pediatric Quality of Life Inventory (PedsQL)

Sagittal synostosis (SS)

Declarations

Ethical Approval and Consent to participate:

The study was approved by the Gothenburg Ethics Committee (no. 856-13) and conducted according to principles in the Declaration of Helsinki. Written consent from children and parents.

Consent for publication:

Written consent from children and parents.

Availability of supporting data:

The manuscript has associated data in a data repository.

Competing interests:

The authors declare no conflicts of interest.

Funding:

Non applicable.

Authors' contributions:

All authors have contributed equally in finalizing the manuscript (literature search, study design, data collection, data analysis, data interpretation, writing).

Acknowledgements:

Non applicable.

References

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